Reza Shakeri - Pediatric Cardiology Fellowship, Modarres Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran.
Abdolrahim Ghasemi - Associate Professor of Pediatric Cardiology, School of Medicine, Shahid Modarres Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Ramin Baghaei Tehrani - Associate Professor of Cardiac Surgery, School of Medicine, Shahid Modarres Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Background This study aimed to investigate diverse structure of pulmonary blood vessels in patients with combined pulmonary atresia and ventricular septal defect and examine the effect on surgery result. Materials and Methods This cohort retrospective study was conducted on admittances to Tehran and Sari Hospitals at 2005-2016 for pulmonary atresia with ventricular septal defect (PA-VSD) with pulmonary artery (PA) anatomical variation. Diagnosis of pulmonary blood vessels in patients with PA-VSD was based on the results of CT Angiography (CTA), MRI, or cardioangiography examinations. Patients were followed up clinically by means of imaging in the course of initial repair, pre-definitive surgery preparation, and post-operative stages. All data sets were analyzed using Stata software version 13.0. Results Abundancy of PA-VSD classes A, B, and C was 34 (37.8%), 51 (56.7%), and 5 (5.6%) individuals respectively, while mean age and standard deviation of subjects for each of the said groups was 166.5±75.6, 135.79±5.3, and 108.4±152 months respectively. 38 (42.2%) were 1-year or younger at the time of initial surgery. As regards between-class age distribution, 18 (52.9%) individuals in group A, 20 (39.2%) individuals in group B, and the entire 5 individuals in group C were collectively above the age of 1 years old. Conclusion In view of the recent studies and the present investigation involving 90 patients with PA-VSD, it is easy to apprehend the large-scale applicability of complete-repair surgical interventions for patients suffering from this disease. This holds true specifically for class A patients who enjoy a longer lifespan and a more favorable prognosis.

Children, Pulmonary Atresia, Pulmonary Blood Vessels, Ventricular Septal Defect